Patterns of clinical and imaging presentations in patients with spontaneous intracranial hypotension due to spinal cerebrospinal fluid venous fistula: A single-center retrospective cross-sectional study.

OBJECTIVE: To identify distinct clinical or imaging subtypes of spontaneous intracranial hypotension (SIH) due to spinal cerebrospinal fluid (CSF) venous fistula (CVF). BACKGROUND: Spontaneous intracranial hypotension is classically understood to present clinically with an orthostatic headache and stereotyped brain magnetic resonance imaging (MRI) findings; however, most prior literature examining clinical and brain MRI features of SIH has focused on all types of spinal CSF leaks concurrently. This study aimed to evaluate whether data support the possibility of internally consistent subtypes based on brain imaging features and clinical symptoms analogous to those seen in primary headache syndromes. METHODS: This retrospective cross-sectional single-institution study included 48 consecutive patients meeting the International Classification of Headache Disorders, 3rd edition criteria for SIH due to CVF. Clinical symptoms, pre-treatment brain MRI, and symptom duration were analyzed. Clinical and MRI data were analyzed to identify patterns and associations between symptoms and imaging findings. RESULTS: A total of 20 males and 28 females were evaluated, with a mean (standard deviation) age of 61 (10) years. In all, 44/48 (92%) patients experienced headaches, though 18/48 (40%) did not endorse relief when flat, including six of the 48 (13%) with worsening symptoms when flat. In all, 19/48 (40%) patients reported at least one migraine symptom, and six of the 48 (13%) presented with at least one migraine symptom and had no relief when flat. Clinical symptoms clustered primarily into a "classic" presentation consisting of relief when flat, occipital head pain, comorbid neck pain, a pressure/throbbing headache quality, and an "atypical" presentation that was characterized by having several differences: less relief when flat (nine of 22 (41%) vs. 20/23 (87.0%), p = 0.002; odds ratio [OR] 0.110, 95% confidence interval [CI] 0.016-0.53), more frontal head pain (14/22 (64%) vs. one of 23 (4%), p < 0.001; OR 35.0, 95% CI 4.2-1681.0), less neck pain (two of 21 (4.5%) vs. nine of 13 (69.6%), p < 0.001; OR 0.023, 95% CI 0.0005-0.196), and more stabbing/sharp headache quality (nine of 22 (41%) vs. two of 23 (9%), p = 0.017; OR 7.0, 95% CI 1.18-75.9). Brain MRI findings clustered into three groups: those presenting with most imaging findings of SIH concurrently, those with brain sag but less pachymeningeal/venous engorgement, and those with pachymeningeal/venous engorgement but less brain sag. CONCLUSION: This study highlights the clinical and imaging diversity among patients with SIH due to CVF, challenging the reliance on classic orthostatic headache alone for diagnosis. The findings suggest the existence of distinct SIH subtypes based on clinical and imaging presentations, underscoring the need for comprehensive evaluation in patients with suspected CVF. Future research should further elucidate the relationship between clinical symptoms and imaging findings, aiming to refine diagnostic criteria and enhance understanding of SIH's pathophysiology.

Cardiac-to-Bronchial Fistula in Hepatocellular Carcinoma: A Case Report.

Hepatocellular carcinoma (HCC) stands as a significant contributor to cancer-related mortality globally. While the acute and often fatal manifestations of locally advanced HCC primarily present within the abdomen, it is crucial to recognize that the respiratory and circulatory systems can also fall victim due to the liver's unique anatomical position within the body. Here, we present the case of a 63-year-old male recently diagnosed with locally advanced HCC with vascular invasion. Shortly after receiving target therapy and focal radiotherapy, the patient developed repeated secondary infections and a persistent diaphragmatic defect. As the necrotic tissue invaded the pleural space, subsequent tumor-to-bronchial and tumor-to-cardiac fistulas emerged, resulting in an abnormal connection between the respiratory and cardiovascular systems, leading to massive air emboli in circulation. This report highlights the risk of supradiaphragmatic complications in HCC patients with post-treatment secondary infections, particularly in patients predisposed to developing diaphragmatic defects.

Aorto-left atrial fistula secondary to aortic infective endocarditis in a dog with a bicuspid aortic valve.

An 11-year-old male neutered American bulldog was presented for evaluation of thrombocytopenia, acute onset of ataxia, and vomiting. A new murmur was auscultated on physical examination. Transthoracic echocardiographic examination revealed a bicuspid aortic valve, vegetative lesions on the aortic valve, and continuous shunting from the aortic root to the left atrium through an aorta to left atrial fistula. The dog was euthanized due to its guarded prognosis and critical condition. Pathological examination confirmed presence of a bicuspid aortic valve, aorto-left atrial fistula, and aortic infective endocarditis. Antemortem blood culture revealed two unusual organisms: Achromobacter xylosoxidans and Fusobacterium mortiferum.

Identifying Patients with CSF-Venous Fistula Using Brain MRI: A Deep Learning Approach.

BACKGROUND AND PURPOSE: Spontaneous intracranial hypotension is an increasingly recognized condition. Spontaneous intracranial hypotension is caused by a CSF leak, which is commonly related to a CSF-venous fistula. In patients with spontaneous intracranial hypotension, multiple intracranial abnormalities can be observed on brain MR imaging, including dural enhancement, "brain sag," and pituitary engorgement. This study seeks to create a deep learning model for the accurate diagnosis of CSF-venous fistulas via brain MR imaging. MATERIALS AND METHODS: A review of patients with clinically suspected spontaneous intracranial hypotension who underwent digital subtraction myelogram imaging preceded by brain MR imaging was performed. The patients were categorized as having a definite CSF-venous fistula, no fistula, or indeterminate findings on a digital subtraction myelogram. The data set was split into 5 folds at the patient level and stratified by label. A 5-fold cross-validation was then used to evaluate the reliability of the model. The predictive value of the model to identify patients with a CSF leak was assessed by using the area under the receiver operating characteristic curve for each validation fold. RESULTS: There were 129 patients were included in this study. The median age was 54 years, and 66 (51.2%) had a CSF-venous fistula. In discriminating between positive and negative cases for CSF-venous fistulas, the classifier demonstrated an average area under the receiver operating characteristic curve of 0.8668 with a standard deviation of 0.0254 across the folds. CONCLUSIONS: This study developed a deep learning model that can predict the presence of a spinal CSF-venous fistula based on brain MR imaging in patients with suspected spontaneous intracranial hypotension. However, further model refinement and external validation are necessary before clinical adoption. This research highlights the substantial potential of deep learning in diagnosing CSF-venous fistulas by using brain MR imaging.

Ventriculo-gallbladder shunt: case series and literature review.

BACKGROUND: The ventriculoperitoneal shunt (VPS) is the gold-standard surgical technique to treat hypertensive hydrocephalus; however, it may fail in 20 to 70% of cases. The present study shows an alternative for patients with contraindications to VPS. METHODS: A case series of nine patients. The medical records of all patients under 17 years of age who underwent ventriculo-gallbladder (VGB) shunt at a pediatric hospital from January 2014 to October 2022 were reviewed. RESULTS: There were 6 (66.7%) males and 3 (33.3%) females. The average age of 73.6 months or 6.1 years at the time of surgery. They had undergone, on average, 5.1 VPS reviews before the VGB shunt. Five (55.5%) had complications of VGB shunt: infection (11.1%), atony (11.1%), hypodrainage (11.1%), and ventriculoenteric fistula (22.2%); all these patients got better at surgical reapproach, and in two of them, the VGB shunt was re-implanted. CONCLUSION: This case series shows a lower risk of death and a similar risk of complications compared to other alternative shunts. This article spotlighted VGB as a viable alternative when VPS fails or has contraindications.

Dermatologic manifestations in pediatric patients with inflammatory bowel disease.

BACKGROUND: Despite studies of dermatologic manifestations in adults with inflammatory bowel disease (IBD), little is known about the prevalence of IBD-associated skin lesions and their correlation with IBD severity in children. We aimed to address these knowledge gaps in our single-center cohort of children with IBD. METHODS: Retrospective chart review of 528 children and adolescents (≤18 years old) with IBD and seen at Mayo Clinic (Rochester, MN) between 1999 and 2017 was conducted. The Chi-Square/Fischer's exact test (with p ≤ .05 to signify statistical significance) was applied to compare categorical outcomes between Crohn's disease (CD) and ulcerative colitis (UC) patients. RESULTS: In total, 425 IBD patients (64.9% CD, 53% males) and ≥1 dermatologic diagnosis were included. Presence of ≥1 cutaneous infection was recorded in 42.8% of participants. Acne was the most common non-infectious dermatologic condition (30.8%), followed by eczema (15.8%) and perianal skin tags (14.6%). Angular cheilitis (p = .024), keratosis pilaris (KP, p = .003), and perianal skin complications (i.e., skin tags, fistula, and abscesses; all p < .001) were more frequently diagnosed among children with CD, while fungal skin infections (p = .017) were more frequently diagnosed in UC patients. Severity of IBD correlated with higher prevalence of perianal fistula (p = .003), perianal abscess (p = .041), psoriasis (p < .001), and pyoderma gangrenosum (PG, p = .003). CONCLUSIONS: Both IBD-specific and IBD-nonspecific dermatologic conditions are very prevalent in childhood IBD, the most common being infectious. Children with CD are more likely to experience angular cheilitis, KP, and perianal skin findings than those with UC. Perianal disease, psoriasis, and PG are associated with more severe IBD.

Urinary catheter placement and adverse urinary outcomes with a focus on elevated risk in men with indwelling Foley catheters.

BACKGROUND: Prior studies indicated a link between urinary catheter use and urinary complications, highlighting the need for comprehensive, gender-specific investigations. This study explored the association through a national retrospective cohort, emphasizing gender disparities and long-term outcomes. METHODS: Our study utilized data from the entire population covered by Taiwan's National Health Insurance Research Database from 2000 to 2017. We included 148,304 patients who had undergone Foley catheter placement and their propensity-scores matched controls in the study. We evaluated urinary complications, which encompassed urinary tract cancer, urolithiasis, urethral stricture, obstructive uropathy, reflux uropathy, fistula, diverticulum, caruncle, false passage, prolapsed urethral mucosa, urinary tract rupture, and urinary tract infection. These were assessed using the Fine and Gray sub-distribution proportional hazards model to compare between the Foley and non-Foley groups. Sensitivity analyses were conducted with different matching ratios. RESULTS: In the study, the non-Foley group presented a marginally higher mean age (75.24 ± 10.47 years) than the Foley group (74.09 ± 10.47 years). The mean duration of Foley catheterization was 6.1 ± 4.19 years. Men with Foley catheterization exhibited the highest adjusted sub-distribution hazard ratios for urinary tract cancer (6.57, 95% CI: 5.85-7.37), followed by women with Foley catheterization (4.48, 95% CI: 3.98-5.05), and men without catheterization (1.58, 95% CI: 1.39-1.8) in comparison with women without the procedure. Furthermore, men with Foley catheterization were found to be at the greatest risk for complications such as urolithiasis, urethral stricture, obstructive and reflux uropathy, fistula, diverticulum, caruncle, false passage, prolapsed urethral mucosa, and urinary tract rupture. Conversely, women with urinary catheterization were most susceptible to urinary tract infections. CONCLUSIONS: The evidence confirms that urinary catheterization significantly increases urinary complications, particularly among men. Our study underscores the crucial need for healthcare providers to carefully evaluate the necessity of catheterization, aim to shorten its duration whenever feasible, and strictly adhere to established protocols to minimize complications.

Multiple Lymphaticovenular Anastomoses for Chyluria in Klippel-Trenaunay Syndrome.

ABSTRACT: Klippel-Trenaunay syndrome (KTS) is characterized by port-wine stains, mixed vascular malformations, and soft tissue and bone hypertrophy. Klippel-Trenaunay syndrome is occasionally complicated by chyluria, for which there is no effective treatment currently. We report a case of KTS complicated by intractable chyluria and hematuria due to a lymphatic-ureteral fistula. The patient was successfully treated with multiple lymphaticovenular anastomoses (LVAs).A 66-year-old woman with an enlarged left lower extremity since childhood was diagnosed with KTS. At 60 years of age, she developed chyluria (urine albumin, 2224 µg/mL) and hematuria. Lymphoscintigraphy showed a lymphatic-ureteral fistula near the ureterovesical junction. Conservative treatment was ineffective. She also developed left lower extremity lymphedema, which gradually worsened. Leg cellulitis and purulent pericarditis developed because of hypoalbuminemia (minimum serum albumin level, 1.3 g/dL).We performed 14 LVAs in 2 surgeries to reduce lymphatic fluid flow through the lymphatic-ureteral fistula. The chyluria and hematuria resolved soon after the second operation, and the urine albumin level decreased (3 µg/mL). After 28 months, she had no chyluria or hematuria recurrence and her serum albumin level improved (3.9 g/dL). Multiple LVAs can definitively treat chyluria caused by a lymphatic-ureteral fistula in patients with KTS.

Management of cervical CSF-venous fistula causing acute cognitive impairment and coma.

CSF-venous fistulas (CVFs) are increasingly recognised as a cause of spontaneous intracranial hypotension. They may present atypically including with brain sagging pseudo-dementia. Cervical CVFs are rare and their management can be difficult due to associated eloquent nerve roots. We report the case of a 49-year-old woman who presented with cognitive decline progressing to coma. Brain imaging showed features of spontaneous intracranial hypotension and a right C7 CVF was identified at digital subtraction and CT myelography. Initial treatment with CT-guided injection of fibrin sealant produced temporary improvement in symptoms before surgical treatment resulted in total clinical remission and radiological resolution.

Treating a Bypass with Bypass Surgery: Repair of a Duodeno-sigmoid Fistula Complicating Acute Diverticulitis.

Diverticular disease is common in Western countries; one-third of patients with diverticular disease develop diverticulitis during their lifetime of whom 5% may experience serious complications. We describe a rare complication of diverticulitis: a duodeno-colic fistula in a patient with an elongated sigmoid colon (dolicosigma). The patient complained of abdominal pain, diarrhea, weight loss, and feculent vomiting. Radiological studies and gastroscopy demonstrated a fistula between the second portion of the duodenum and the sigmoid colon. Curative surgery cured the fistula and completely resolved its associated signs and symptoms.

Characterization of long-term survival in Medicare patients undergoing arteriovenous hemodialysis access.

BACKGROUND: Patients undergoing arteriovenous (AV) access creation for hemodialysis often have significant comorbidities. Our goal was to quantify the long-term survival and associated risks factors for long-term mortality in these patients to aid in optimization of goals and expectations. METHODS: The Vascular Implant Surveillance and Interventional Outcomes Network Vascular Quality Initiative Medicare linked data was used to assess long-term survival in the HD registry. Demographics, comorbidities, and interventions were recorded. Because the majority of hemodialysis patients are provided Medicare, Medicare linkage was used to obtain survival data. Multivariable analysis was used to identify independent associations with mortality. RESULTS: There were 13,945 AV access patients analyzed including 10,872 (78%) AV fistulas and 3073 (22%) AV grafts. The median age was 67 years and 56% of patients were male. Approximately one-third had a prior AV access and 44.7% had prior tunneled dialysis catheters. Patients receiving an AV fistula, compared with AV grafts, were more often younger, male, White, obese, independently ambulatory, preoperatively living at home, and less often have a prior AV access and tunneled dialysis catheters (P < .05 for all). The 5-year mortality overall was 62.9% with 61.2% for AV fistulas and 68.8% for AV grafts (P < .001). On multivariable analysis for 5 year mortality, nonambulatory status (hazard ratio [HR], 1.67; 95% confidence interval [CI], 1.53-1.83; P < .001), lower extremity access (HR, 1.67; 95% CI, 1.35-2.05; P < .001), human immunodeficiency virus or acquired immunodeficiency syndrome (HR, 1.44; 95% CI, 1.13-1.82; P < .001), White race (HR, 1.43; 95% CI, 1.35-1.51; P < .001), congestive heart failure (HR, 1.33; 95% CI, 1.26-1.41; P < .001), chronic obstructive pulmonary disease (HR, 1.23; 95% CI, 1.15-1.31; P < .001), and AV graft placement (HR, 1.12; 95% CI, 1.02-1.23, P = .016) were most associated with poor survival. Factors associated with improved survival were never smoking (HR, .73; 95% CI, 0.67-0.79; P < .001), prior/quit smoking (HR, .78; 95% CI, 0.72-0.84; P < .001), preoperative home living (HR, .75; 95% CI, 0.68-0.83; P < .001), and hypertension (HR, .89; 95% CI, 0.8-0.99; P = .03). CONCLUSIONS: Long-term survival in Medicare patients undergoing AV access creation is poor with nearly two-thirds of patients having died at 5 years. There are many modifiable risk factors that may improve survival in these patients and give an opportunity for transplantation.

Endovascular embolization of cerebrospinal fluid-venous fistula: a comprehensive systematic review on its efficacy and safety for the management of spontaneous intracranial hypotension.

Cerebrospinal fluid (CSF)-venous fistula can cause spontaneous intracranial hypotension (SIH) and poses a significant diagnostic and management challenge. This study aims to provide a comprehensive overview of the clinical and radiological outcomes of endovascular embolization as a novel treatment approach for CSF-venous fistula in patients with SIH. This systematic review adheres to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses 2020 statement. The primary outcome was the efficacy of the embolization procedure in occlusion of the CSF-venous fistula, and secondary outcomes included procedural complications and improvement of clinical symptoms and radiological findings. A total of nine studies consisting of 77 patients met the inclusion criteria. Orthostatic and/or Valsalva headache was the most common symptom. The mean age of the patients was 57 ± 8.9 years, and females accounted for 59.7% (46/77) of the cases. Sixty-five (84.4%) patients reported complete resolution or significant improvement in symptoms. The Bern score, Headache Impact Test-6, and the Patient Global Impression of Change scales demonstrated significant improvements in radiological findings and patients' quality of life. Following the procedure, 22 patients (28.6%) experienced rebound intracranial hypertension and 27 patients (35.1%) had transient local pain at the site of the embolization. Our study showed that endovascular embolization is a safe and effective treatment for CSF-venous fistula in patients with SIH, providing complete resolution or significant improvement of clinical symptoms and radiological findings, and positive impacts on patients' quality of life.

Combined Cystoscopic-Abdominal Versus Abdominal-Only Route for Complete Excision of Large Deep Endometriosis Nodules Infiltrating the Supratrigonal Area of the Bladder: A Comparative Study.

STUDY OBJECTIVE: Surgical excision of large deep endometriosis nodules infiltrating the bladder may be challenging, particularly when the nodule limits are close to the trigone and ureteral orifice. Bladder nodules have classically been approached abdominally. However, combining a cystoscopic with an abdominal approach may help to better identify the mucosal borders of the lesion to ensure complete excision without unnecessary resection of healthy bladder. This study aimed to compare classical excision of large bladder nodules by abdominal route with a combined cystoscopic-abdominal approach. DESIGN: Retrospective comparative study on data prospectively recorded in a database. Patients were managed from September 2009 to June 2022. SETTING: Two tertiary referral endometriosis centers. PATIENTS: A total of 175 patients with deep endometriosis infiltrating the bladder more than 2 cm undergoing surgical excision of bladder nodules. INTERVENTIONS: Excision of bladder nodules by either abdominal or combined cystoscopic-abdominal approaches. MEASUREMENTS AND MAIN RESULTS: A total of 141 women (80.6%) were managed by abdominal route and 34 women (19.4%) underwent a combined cystoscopic-abdominal approach. In 99.4% of patients, the approach was minimally invasive. Patients with nodules requiring the combined approach had a lower American Fertility Society revised score and endometriosis stage and less associated digestive tract nodules, but larger bladder nodules. They were less frequently associated with colorectal resection and preventive stoma. Operative time was comparable. The rate of early postoperative complications was comparable (8.8% vs 22%), as were the rates of ureteral fistula (2.2% vs 2.9%), bladder fistula (2.2% vs 0), and vesicovaginal fistula (0.7% vs 2.9%). CONCLUSION: In our opinion, the combined cystoscopic-abdominal approach is useful in patients with large bladder nodules with limits close to the trigone and ureteral orifice. These large deep bladder nodules seemed paradoxically associated to less nodules on the digestive tract, resulting in an overall comparable total operative time and complication rate.

Posterior median surgical approach to anterior sacral meningocele complicated by rectothecal fistula.

In this case report, we aimed to describe the clinical presentation, surgical approach, and follow-up of a patient with rare anterior meningocele associated with rectothecal fistula. An 17-year-old female patient was admitted to the emergency department with meningitis. On further examinations, an anterior sacral meningocele accompanied by rectothecal fistula was detected. Appropriate antibiotic treatment was arranged and surgical plan was made with the pediatric surgery clinic. The patient underwent meningocele repair via posterior approach and colostomy operation. The patient did not experience any neurological issues after the surgery. The colostomy was reversed 3 months later, and third-month follow-up MRI showed complete regression of the meningocele sac with no neurological complications. Anterior meningocele accompanied by a rectothecal fistula is a rare and complicated case. Only seven cases of coexisting ASM and RTF have been reported in literature. Although both anterior and posterior approaches have been used for the treatment of ASM, the choice of treatment is essentially based on the patient's clinical and imaging findings.

Cerebrospinal fluid-lymphatic fistula in a child with generalized lymphatic anomaly treated with targeted blood patch - a rare case report and review of the literature.

Spontaneous intracranial hypotension may result in debilitating postural headaches and severe neurological symptoms due to secondary cerebellar sagging. The most common cause is the cerebrospinal fluid (CSF) leak within the spinal canal. Although previously reported in only a few cases, also paraspinal lymphatic malformations causing vertebral bone destruction may occasionally result in CSF leak to these pathological formations. Here, we present a case of a 9-year-old girl with generalized lymphatic anomaly (GLA) presenting with severe postural headache. Radiological imaging revealed a typical feature of cerebellar sagging. Myelography localized the CSF leakage into vertebral bodies of C7 and Th1, which both were partly involved in pathological paravertebral masses of known lymphatic anomaly, and from there along the right C8 nerve root sleeve into the anomaly. As the C8-nerve root could not be ligated due to the risk of significant neurological injury, we attempted image-guided targeted percutaneous epidural placement of a blood patch directly into the foramen at the affected level. The procedure resulted in obliteration of the fistula and regression of cerebellar sagging, with significant relief of symptoms. Although it is an extremely rare coincidence, patients with paraspinal lymphatic malformations may develop intraspinal CSF leak into these pathological formations. The present case report suggests that besides a direct surgical obliteration of the fistula and sacrificing the nerve root, a targeted percutaneous epidural blood patch may be a possible alternative in the case of a functionally important nerve root.

CSF-Venous Fistula of the Clival Skull Base: A Unique Case Study and Literature Review.

An adolescent male presented with orthostatic headaches following head trauma. MRI showed cerebellar tonsil displacement and a bony defect in the clival skull base. Digital subtraction myelography (DSM) confirmed a cerebrospinal fluid-venous fistula (CVF). This was repaired endoscopically. CVFs cause uncontrolled flow of CSF into the venous system resulting in symptoms of intracranial hypotension. They're often difficult to identify on initial imaging. This is the first reported CVF originating in the central skull base, and the first treated via endoscopic trans-nasal approach. CVFs may elude initial imaging, making DSM crucial for unexplained spontaneous intracranial hypotension. Laryngoscope, 134:645-647, 2024.